Abstract：
BACKGROUND: Stiff skin syndrome (SSS) is a rare disease characterized by thickened, indurated skin and limited joint movement. Multiple diverse phenotypes have been reported, and the correlation of severity with the clinical heterogeneity and histopathological findings of SSS needs to be refined.
OBJECTIVE: To define subtypes based on clinical features and predict the prognosis of a new SSS classification.
METHODS: Eighty-three patients with SSS were retrospectively reviewed for clinicopathological manifestations and routine laboratory workup, including 59 cases obtained from a PubMed search between 1971 and 2022 and 24 cases diagnosed in our department between 2003 and 2022.
RESULTS: Among the 83 patients, 27.7, 41, and 31.3% had classic widespread, generalized segmental, and localized SSS, respectively. Joint immobility was present in 100, 71, and 20% of classic, generalized, and localized cases, respectively. Histopathologic findings were common among the 3 groups, and based on that, we further found a difference in the distribution of proliferative collagen. 54.5% of classic and 50% of generalized cases occurred throughout the dermis or the subcutis, whereas 76% of localized cases were mainly involved in the reticular dermis or subcutis. In patients with incipient localized SSS, 42% (21/50) developed generalized SSS, and only 6% (3/50) progressed to classic SSS, whereas more than half of the incipient generalized SSS cases (60.6%, 20/33) developed classic SSS.
CONCLUSIONS: This retrospective study was limited to previously published cases with limited data.
CONCLUSIONS: We propose a distinct clinical classification characterized by lesion distribution, including classic widespread, generalized segmental, and localized SSS, associated with disease severity and prognosis.
OBJECTIVE: To define subtypes based on clinical features and predict the prognosis of a new SSS classification.
METHODS: Eighty-three patients with SSS were retrospectively reviewed for clinicopathological manifestations and routine laboratory workup, including 59 cases obtained from a PubMed search between 1971 and 2022 and 24 cases diagnosed in our department between 2003 and 2022.
RESULTS: Among the 83 patients, 27.7, 41, and 31.3% had classic widespread, generalized segmental, and localized SSS, respectively. Joint immobility was present in 100, 71, and 20% of classic, generalized, and localized cases, respectively. Histopathologic findings were common among the 3 groups, and based on that, we further found a difference in the distribution of proliferative collagen. 54.5% of classic and 50% of generalized cases occurred throughout the dermis or the subcutis, whereas 76% of localized cases were mainly involved in the reticular dermis or subcutis. In patients with incipient localized SSS, 42% (21/50) developed generalized SSS, and only 6% (3/50) progressed to classic SSS, whereas more than half of the incipient generalized SSS cases (60.6%, 20/33) developed classic SSS.
CONCLUSIONS: This retrospective study was limited to previously published cases with limited data.
CONCLUSIONS: We propose a distinct clinical classification characterized by lesion distribution, including classic widespread, generalized segmental, and localized SSS, associated with disease severity and prognosis.
摘要：
背景：皮肤僵硬综合征（SSS）是一种罕见的疾病，其特征是增厚，皮肤硬结和关节运动受限。已经报道了多种不同的表型，严重程度与SSS临床异质性和组织病理学结果的相关性有待完善。
目的：根据临床特征定义亚型并预测新的SSS分类的预后。
方法：回顾性分析83例SSS患者的临床病理表现和常规实验室检查，包括1971年至2022年从PubMed搜索获得的59例病例，以及2003年至2022年在我们部门诊断的24例病例。
结果：在83例患者中，27.7、41和31.3%有经典的广泛传播，广义分段，和本地化的SSS，分别。关节不活动存在于100％，71％和20％的经典，广义的，和局部病例，分别。组织病理学发现在3组中是常见的，基于此,我们进一步发现增殖胶原的分布存在差异。54.5%的经典病例和50%的全身病例发生在整个真皮或皮下组织，而76％的局部病例主要累及网状真皮或皮下组织。在早期局部SSS患者中，42%(21/50)开发了广义SSS，只有6%(3/50)发展为经典SSS，而超过一半的早期广泛性SSS病例(60.6%，20/33）开发了经典的SSS。
结论：这项回顾性研究仅限于以前发表的数据有限的病例。
结论：我们提出了一种以病变分布为特征的独特临床分类，包括经典的广泛传播，广义分段，和本地化的SSS，与疾病严重程度和预后相关。
目的：根据临床特征定义亚型并预测新的SSS分类的预后。
方法：回顾性分析83例SSS患者的临床病理表现和常规实验室检查，包括1971年至2022年从PubMed搜索获得的59例病例，以及2003年至2022年在我们部门诊断的24例病例。
结果：在83例患者中，27.7、41和31.3%有经典的广泛传播，广义分段，和本地化的SSS，分别。关节不活动存在于100％，71％和20％的经典，广义的，和局部病例，分别。组织病理学发现在3组中是常见的，基于此,我们进一步发现增殖胶原的分布存在差异。54.5%的经典病例和50%的全身病例发生在整个真皮或皮下组织，而76％的局部病例主要累及网状真皮或皮下组织。在早期局部SSS患者中，42%(21/50)开发了广义SSS，只有6%(3/50)发展为经典SSS，而超过一半的早期广泛性SSS病例(60.6%，20/33）开发了经典的SSS。
结论：这项回顾性研究仅限于以前发表的数据有限的病例。
结论：我们提出了一种以病变分布为特征的独特临床分类，包括经典的广泛传播，广义分段，和本地化的SSS，与疾病严重程度和预后相关。
